Article No. 440
11 June 2021
Sometimes aHUS alliances Global Action sees things written about aHUS on the internet which are not clear or wrong. Unintentionally misinforming or misleading. Seldom are such answers as those below seen but they can happen.,
There is little awareness of atypical HUS, it is not heard of.
Not entirely true , drop the “atypical” and HUS is something many people would be aware of and heard about but do not know they have. Ask them if they have heard in the news of people dying from eating undercooked meat in dodgy hamburgers, or petting infected animals in mini zoos, or eating salad washed in contaminated water and they will know of E.coli outbreaks and that they can be serious. Tell them it is serious because of HUS which is the cause of victims’ kidney failure and/or death. Add back the atypical and say this is a related illness but not resulting from E.coli poisoning but from other triggers. It is just as serious, if not more.
Typical HUS patients have a genetic marker or a trigger. atypical HUS has no known cause.
Typical HUS patients have E.Coli poisoning as the cause of their illness with no genetic involvement. atypical HUS patients have a part of the immune system causing “friendly fire” to the patient because of genetic defects in their control of a part of their immune system. A good thing has gone bad and it is the patient’s own body that is the cause. Bad things are happening in the blood and bad things will happen to the kidneys as a result.
Colds and flu and food poising risk the triggering of a potential fatal episode of aHUS.
Virus and bacterial infections can trigger an aHUS patient’s’uncontrollable immune reaction but so can other things like pregnancy and kidney transplants to name but two. Throughout their lives those who can get aHUS can have many hits, hundreds of hits and the disease does not start. But one of the hits is enough to do so, but it is not always fatal these days if it does breakthrough.
aHUS not been identified for long
.aHUS has been around in some families for centuries. Nearly 100 years ago it was described as a “clumping” disorder, clumps of blood were found in those who died . Nearly 70 years ago it was given a name HUS, Hemolytic Uremic Syndromes. The name describes that something bad is happening in the patient’s blood and kidneys.
The atypical term was added about 30 years ago to keep it and E,coli HUS separate.
25 years ago it was found that a part of the immune system called Complement was the source of the problem. Complement even today is rarely understood by doctors , so do not feel alone. By most rare diseases standards, it has been identified for a long while, as have been the genetic causes that lead to Complement’s friendly fire.
There is not much information about it.
Today there is an enormous amount of information about aHUS from the thousands of people who have researched or treated it, to the stories of patients of who had it around the world. In the past decade many patient websites have sprung up where patients provided information to other patients in their own language and terms. The internet has enabled an explosion of readily accessed articles and blogs. Those who think there is not much information on aHUS in the 2020s are mistaken. Indeed it is impossible to read everything there is to be read about aHUS. This website has a 360 degree view of aHUS not just posts and pages about the authors’ own experience. There is much to learn from others to truly understand what happened to the self.
The main symptom of aHUS is fatigue.
No symptom is experienced by all aHUS patients. Most symptoms may be experienced by less than half of all patients. Only feeling sick and vomiting is reported by more than half of patients When they are very ill, few patients mention fatigue. They have no energy as their red blood cells which carry oxygen around the body are destroyed cells but are so laid low by all their symptoms together they are not going to be active anyway.
Once their aHUS has come under control and a wellness returns patients take time to get their fitness back. That is something which happens with many illnesses. Take COVID survivors mentioning its lingering effects. “Long COVID” as it has become, Well some aHUS patients too, who have had a similar experience, may experience “Long aHUS”.
There are only two treatments for aHUS.
There are two drugs now which can stop the self harm that aHUS patients experience. There are many more treatments, which most aHUS patients need and rely on, especially aHUS patients in countries without access to the two drugs.
People only get drug infusions every two weeks if their blood numbers out of sorts.
Infusions take place at one ( in build up phase) , two, three or four week intervals irrespective of what their blood results are before each infusion. Drug infusions will be repeated until the clinician and patient decide it could be time to stop them as the patient might be in remission.
Infusions take place in a cancer centre.
Possibly, in very few instances if the treating physician is a cancer expert ( cancer can trigger aHUS) or a blood expert. Otherwise it is more likely to be done at a generic infusion centre or a kidney/dialysis unit of a hospital. Even more likely it is done at home, with the patient taking on some of the responsibility for linking themselves up to the infusion bag.
Hard to tell what will happen to patients in time.
Most patients will get back to the health state they had before it all begun. Those with kidney failure will end up on dialysis and might in time get a kidney transplant. Enough is known these days to predict what will happen to patients and their chances of a remission or relapse based on age ,gender, what triggered their aHUS , their genetic make up, and the time that passes. As always with any illness more evidence is being found to help guide such predictions.
There are well under a thousand aHUS patients in the USA
No one knows how many aHUS patients there are anywhere, no such record is kept. The usual estimate for the USA is between 600 and 700 patients based on an out of date assumption from more than 10 years ago. The USA population has grown since then, the disease is better known among clinicians , and patients can access new more effective treatments. Research today would find that the number of aHUS patients in the USA could exceed 2000 and might be as high as 3000 and each year around 150 more patients add to the tally, that would be 750 more in the past five years. Even then aHUS is still a very rare disease.
The aHUS alliance Global Action website has just passed its fifth anniversary. It has been a source for aHUS patients all around the world who look for information about aHUS and find it. Mostly it is internet search engines that determine that. Unless clinicians have been invited to do so , everything on the site is written by non clinicians, just patients or carers who volunteer their time.
Patients and carers new to aHUS usually wonder why there is so little awareness about aHUS, which they because of their have become aware of only too well, even if it remains enigmatic to them.
But in a world of over 7.5 billion people, aHUS will only happen to about 3500 new patients each year and it is hardly going to get much attention. Prior to their encounter it is unlikely they too would have taken much notice. Once it happens to them perhaps 35,000 of their friends and family may now have questions about aHUS that may need answering and ripple out awareness.
All of the answers to the answers in this article have been featured in some way on this website in the past five years to help those who seek.
